Highlights
- •“Non-classical” PNSs are more frequent than reported in the literature.
- •“Non-classical” PNSs more frequently present as peripheral neurological syndromes.
- •Diagnosis of “non-classical” PNS is more difficult than of “classical” syndromes.
- •PNS manifestation usually precedes tumor detection.
- •PNS diagnosis allows detecting tumors at an early stage.
Abstract
Objectives
Paraneoplastic neurological syndromes (PNSs) are rare disorders in association with
cancer and sub-divided into “classical” and “non-classical” syndromes according to
a 2004 consensus paper proposed by a panel of PNS experts. “Classical” PNSs are regarded
to account for the vast majority of cases. However, systematic reports on clinical
PNS manifestations are rare. Therefore, we analyzed the spectrum of PNS in our clinic.
Methods
We retrospectively investigated medical records from consecutive patients diagnosed
with definite PNS and serological evidence of well-characterized onconeural antibodies
(anti-Hu, Yo, Ri, CV2/CRMP5, Ma1, Ma2, and amphiphysin) analyzed between 1991 and
2014 in our clinic.
Results
Of the 50 patients identified with onconeural antibody-positive PNS, 28 patients (56.0%)
had “classical” PNS, and 22 (44.0%) “non-classical” PNS. Subacute cerebellar degeneration
was the most frequent “classical” syndrome, brainstem encephalitis and subacute sensorimotor
neuronopathy the most frequent “non-classical” syndromes. Anti-Hu antibodies were
most frequent in both groups. 86.1% of patients developed neurological symptoms before
the cancer was known. No differences between “classical” and “non-classical” syndromes
were detected with respect to age, tumor entities and median time to diagnosis. However,
whereas most patients with “classical” syndromes were females, there was no gender
predominance in patients with “non-classical” PNS and the latter had significantly
more frequent peripheral neurological syndromes.
Conclusions
The so-called “non-classical” PNSs in association with well-characterized onconeural
antibodies were more common in our patient population than expected. Therefore, in
neurological disorders of unclear etiology with a subacute onset and atypical presentation
further diagnostic work-up including investigation of onconeural antibodies is necessary.
Keywords
To read this article in full you will need to make a payment
Purchase one-time access:
Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online accessOne-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:
Subscribe to Journal of the Neurological SciencesAlready a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
References
- Paraneoplastic syndromes of the CNS.Lancet Neurol. 2008; 7: 327-340
- Recommended diagnostic criteria for paraneoplastic neurological syndromes.J. Neurol. Neurosurg. Psychiatry. 2004; 75: 1135-1140
- Paraneoplastic neurological syndromes and autoimmune encephalitis.Nervenarzt. 2014; 85: 485-498
- Paraneoplastic neurological syndromes — patients' cohort profile in the Czech Republic.Acta Neurol. Scand. 2001; 104: 72-77
- A follow up study of patients with paraneoplastic neurological disease in the United Kingdom.J. Neurol. Neurosurg. Psychiatry. 2004; 75: 1411-1415
- Paraneoplastic neurologic syndrome in the PNS Euronetwork Database.Arch. Neurol. 2010; 67: 330-335
- Paraneoplastic neurological syndromes.Curr. Opin. Neurol. 2012; 25: 795-801
- Positivity of serum “classical” onconeural antibodies in a series of 2063 consecutive patients with suspicion of paraneoplastic neurological syndrome.J. Neuroimmunol. 2013; 259: 75-80
- Screening for tumours in paraneoplastic syndromes: report of an EFNS Task Force.Eur. J. Neurol. 2011; 18: 1-17
- Update on paraneoplastic neurologic disorders.Commun. Oncol. 2010; 7: 219-224
- Paraneoplastic antibodies coexist and predict cancer, not neurological syndrome.Ann. Neurol. 2004; 56: 715-719
- Onconeural antibodies in patients with neurological symptoms: detection and clinical significance.Acta Neurol. Scand. 2011; 124: 83-88
- The spectrum of antineuronal autoantibodies in a series of neurological patients.J. Neurol. Sci. 2004; 220: 29-36
- Anti-Ma and anti-Ta associated paraneoplastic neurological syndromes: 22 newly diagnosed patients and review of previous cases.J. Neurol. Neurosurg. Psychiatry. 2008; 79: 767-773
- Onconeural antibodies in sera from patients with various types of tumours. 58. 2009: 1795-1800
Article info
Publication history
Published online: March 24, 2015
Accepted:
March 16,
2015
Received in revised form:
March 10,
2015
Received:
January 12,
2015
Identification
Copyright
© 2015 Elsevier B.V. Published by Elsevier Inc. All rights reserved.
